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CBL Year 2 > Anaemia > Flashcards

Anaemia Flashcards

1
Q

Basics of what blood does?

A

supply O2 to tissues
supply of nutrients (e.g. glucose, amino acids, and fatty acids)
removal of waste such as CO2, urea and lactic acid
immunological functions (white cells and immunoglobulins)
coagulation
messenger functions (e.g. transport of hormones, signalling of tissue damage)

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2
Q

Composition of blood

A

plasma (55%) - includes water, proteins and other solutes
cells (45%) - platelets, red blood cells, white blood cells (including - lymphocytes, monocytes, granulocytes - basophils, neutrophils and eosinophils)

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3
Q

red blood cell function

A

responsible for transporting oxygen from your lungs to your body’s tissues

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4
Q

lymphocytes function

A

determine the specificity of the immune response to infectious microorganisms and other foreign substances.
- type of white blood cell

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5
Q

granulocyte function

A

Granulocytes work together to rid your body of infection or allergens. Each type of granulocyte has its own combination of chemicals and enzymes in its granules

-type of white blood cell

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6
Q

basophil function

A

they have the ability to help detect and destroy some early cancer cells. Another important function of basophils is that they release the histamine in their granules during an allergic reaction or asthma attack

  • type of granulocyte, which is a type of white blood cell
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7
Q

eosinophil function

A

movement to inflamed areas, trapping substances, killing cells, anti-parasitic and bactericidal activity, participating in immediate allergic reactions, and modulating inflammatory responses.

-type of granulocyte, which is a type of white blood cell

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8
Q

hematopoiesis

A

process of making blood cells

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9
Q

FBC

A

full blood count

check the types and numbers of cells in your blood, including red blood cells, white blood cells and platelets.

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10
Q

describe reticulocyte count

A

a blood test that measures the amount of these cells in the blood. In the presence of some anemias, the body increases production of red blood cells (RBCs), and sends these cells into the bloodstream before they are mature.

normal range is from 0.5% to 1.5%

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11
Q

what does a blood film show

A

the evaluation of white blood cells (WBCs, leucocytes), red blood cells (RBCs, erythrocytes), and platelets (thrombocytes)

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12
Q

basic properties of red blood cells (erythrocytes)

A

smooth biconcave disc shape
anucleate
life span: 90-120 days
clearance by the reticuloendothelial system (mainly the spleen)

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13
Q

development of red blood cells (erythrocytes)

A

stimulated by erythropoietin (EPO)
oxygen levels sensed by kidney which modulates EPO production
androgens also increase red cell production

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14
Q

basic properties of platelets

A

small and anucleate
life span: 7-10 days
normal clearance by reticuloendothelial system (mainly the spleen)

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15
Q

Development of platelets

A

stimulated by thrombopoetein (TPO) produced by the liver
TPO increases number / differentiation megakaryocytes
arise through fragmentation of megakaryocytes cytoplasm

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16
Q

function of platelets

A

blood coagulation upon vasculature injury
surface coated with glycoproteins for adhesion/aggregation
bind to fibrinogen for platelet-platelet aggregation
release granules and active clotting cascade

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17
Q

basic properties of neutrophils

A

multi lobed nucleus , granular cytoplasm
lifespan - 6-10 hours
normal clearance by reticuloendothelial system (mainly spleen)

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18
Q

Development of neutrophils

A

mature in bone marrow prior to circulation

require G-CSF (granulocyte colony stimulating factor)

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19
Q

neutrophil function

A

detect microbial invasion via inflammatory mediators
engulf microbes (bacteria) by phagocytosis
kill/digest microbes using reactive oxygen species (H2O2) and enzymes (myeloperoxidases)

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20
Q

basic properties of monocytes

A

largest leucocyte
diverse subsets and functions
life span 1-2 days

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21
Q

development of monocytes

A

require GM - CSF (granulocyte - macrophage colony stimulating factor) and M- CSF (macrophage colony stimulating factor) for development
share common progenitor with granulocyte
develop into macrophages and dendritic cells in tissue

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22
Q

function of monocytes

A

phagocytosis of bacteria/virus
antigen presenting cells
replenish tissue macrophages/dendritic cells

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23
Q

basic properties of lymphocytes

A

small highly specialised subsets; B cells, T cells, NK cells
life span varies depending on subtype

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24
Q

Development of lymphocytes

A

initially develop in the bone marrow

development completed in circulation and secondary lymphoid organs

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25
Q

describe plasma cells

A

derived from mature B cells
reside in bone marrow
produce antibody
eccentric round nucleus, basophilic cytoplasm

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26
Q

What does a full blood count measure

A 
concentration of haemoglobin: Hb
red cell to plasma ratio: Hct or PCV
Cell size (mean cell volume = MCV)
amount of Hb/ RBC 
cell numbers (RBC)
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27
Q

Describe mean cell volume (MCV)

A

indicates red cell volume

normal range: 80-100fL

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28
Q

microcytic anaemia MCV

A

<80fL

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29
Q

normocytic anaemia MCV

A

80-100fL

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30
Q

macrocytic anaemia MCV

A

> 100fL

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31
Q

what does it mean if reticulocytes are increased

A

reduced RBC survival

- haemolysis (premature destruction) or bleeding (loss)

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32
Q

what does it mean if reticulocyte numbers are decreased

A

there is a problem with production

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33
Q

Four components of normal haemostasis

A

blood vessel wall
platelets and von willebrand factor
coagulation factor
fibrinogen

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34
Q

platelets 3 main roles in haemostasis

A
  1. to adhere to subendothelial proteins after vascular damage to initiate haemostasis
  2. activate and aggregate with other platelets
  3. support the activation of coagulation factors
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35
Q

von willebrand factor

A

complex adhesive plasma protein synthesised by vascular endothelium

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36
Q

von willebrand factor main role in haemostasis

A

binds platelet surface proteins to mediate platelet adhesion

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37
Q

endothelial cells in haemostasis

negative regulators - soluble and surface

A

critical negative regulators of haemostasis

  • soluble mediators: prostacyclin, NO
  • surface mediators: endothelial protein c receptor, thrombomodulin, heparans

( Negative feedback occurs when a change in a. variable triggers a response. which reverses the initial change.)

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38
Q

sub-endothelium in haemtostasis

A

contains activators of haemostasis:

-collagen, tissue factors, von willebrand

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39
Q

describe coagulation factors

A

series of inactive zymogen proteins in plasma
assemble into functional ‘tenase’ and ‘protrombinase’ complexes on surface of activated platelets
results in rapid burst conversion of prothrombin (FII) to thrombin

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40
Q

what is fibrinogen

A

complex high molecular weight protein abundant in plasma

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41
Q

2 Main roles of fibrinogen in haemostasis

A
  1. binds platelet surface integrins to mediate platelet aggregation
  2. polymerised by thrombin to form fibrin clot
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42
Q

define haemostasis

A

process to prevent and stop bleeding, meaning to keep blood within a damaged blood vessel.

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43
Q

describe the steps in haemostasis

A

step 1: trigger
- collagen and tissue factors are exposed
- von willebrand factor (vWF) binds collagen
step 2: primary haemostasis
- platelets adhere to vWF-collagen
- platelets activate and aggregate
step 3: thrombin generation
- TF (tissue factor) initiates rapid thrombin generation on activated platelets
step 4: thrombin consolidates clot formation
- thrombin converts fibrinogen to fibrin and completes platelet activation
- stable fibrin- platelet clot is formed

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44
Q

two parts of regulation of homeostasis

A

clot formation

fibrinolysis

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45
Q

describe clot formation in the regulation of homeostasis

A
  • vWF (von willebrand factor) adhesivity is regulated by ADAMTS 13
  • thrombin is regulated by antithrombin and the activated protein C system
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46
Q

describe fibrinolysis in regulation of homeostasis

A
  • thrombin activates the protease plasminogen to plasmin
  • fibrin cleaved by plasmin into fibrin degradation products (incl. D dimer)

Fibrinolysis is a process that prevents blood clots from growing and becoming problematic. Primary fibrinolysis is a normal body process, while secondary fibrinolysis is the breakdown of clots due to a medicine, a medical disorder, or some other cause.

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47
Q

4 core laboratory tests of haemostasis

A
  1. platelet count and blood film
  2. Coagulation screen (including prothrombin time (PT) and activated partial thromboplastin time (APTT))
  3. fibrinogen level
  4. D dimer level
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48
Q

3 things a prolonged PT or/and APTT indicate??

A
  1. reduced levels of coagulation factor(s) (from genetic or acquired bleeding disorders)
  2. Reduced function of coagulation factor(s) (most anticoagulant drugs)
  3. Laboratory artefact ( could be from: wrong blood tube, incompletely filled blood tube, heparin contamination of samples, antiphospholipid syndrome)
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49
Q

what does it mean if just the PT interval is prolonged (what factor/ pathway?)

A

Factor VII
(extrinsic pathway)

This may be caused by conditions such as liver disease, vitamin K deficiency, or a coagulation factor deficiency (e.g., factor VII deficiency).

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50
Q

what does it mean if just APTT interval is prolonged

what factor/pathway?

A

Factors VIII, IX, XI

intrinsic pathway

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51
Q

what does it mean if PT and APTT are both prolonged in terms of factor involved and pathway

A

means multiple factor defects
factors II, V, X, fibrinogen
(common pathway)

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52
Q

what does high fibrinogen levels indicate

A

acute phase response
pregnancy

(is a prominent systemic reaction of the organism to local or systemic disturbances in its homeostasis caused by infection, tissue injury, trauma or surgery, neoplastic growth or immunological disorders)

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53
Q

what is acute phase response

A

group of proteins whose concentrations in blood plasma either increase or decrease due to inflammation

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54
Q

describe how fibrinogen levels are measured

A

plasma levels are usually measured using a clotting assay similar to PT or APTT
normal levels are ~1.5-4.0g/dL

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55
Q

what does low fibrinogen levels indicate

A

some important acquired bleeding disorders

liver disease, massive transfusion, disseminated intravascular coagulation (DIC)

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56
Q

what is DIC

A

disseminated intravascular coagulation

  • serious disorder in which the proteins that control the blood become overactive
  • small blood clots develop throughout the bloodstream, blocking small blood vessels. The increased clotting depletes the platelets and clotting factors needed to control bleeding, causing excessive bleeding.
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57
Q

what causes DIC

A

inflammation in response to an infection, injury or illness
severe tissue damage from burns, or trauma
clotting factors caused by some cancers or pregnancy complications

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58
Q

describe D-dimer level

A

D-dimer is a fibrin degradation product
plasma levels measured by immunoassay
normal levels ~<500ng/mL

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59
Q

3 things that elevated D-dimer indicate

A
  1. DIC (disseminated intravascular coagulation)
  2. VTE (venous thromboembolism)
  3. pregnancy, liver or kidney disease, sepsis, malignancy, inflammation, increasing age
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60
Q

what is Thromboelastography

A

is a viscoelastic hemostatic assay that measures the global viscoelastic properties of whole blood clot formation under low shear stress. TEG shows the interaction of platelets with the coagulation cascade (aggregation, clot strengthening, fibrin cross-linking and fibrinolysis)

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61
Q

some specialist laboratory tests for the blood (factors)

A
  1. levels of coagulation factors
  2. von willebrand factor levels
  3. platelet function tests
  4. Bone marrow morphology
  5. anticoagulant drug levels
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62
Q

5 signs of DVT

A 
leg pain
swelling
tenderness
discolouration
pitting oedema
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63
Q

6 signs of PE

A 
SOB
Cough (haemoptysis)
chest pain
tachycardia
hypotension
low-grade fever
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64
Q

two long term consequences of VTE

A

PTS - post thrombotic syndrome

CTEPH - chronic thromboembolic pulmonary hypertension

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65
Q

what is PTS (post thrombotic syndrome) / describe it

A

-chronic venous stasis with swelling, discomfort, skin -changes, and sometimes skin ulceration
-caused by damage to venous valves by thrombus in DVT
(20-50% of patients develop PTS after DVT)

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66
Q

what is CTEPH (chronic thromboembolic pulmonary hypertension)/describe it

A

chronic breathlessness, hypoxia, and right sided heart failure
caused by obstruction of major pulmonary arteries
(affects up to 9.1% patients who have had symptomatic PE)
lifelong anticoagulant is recommended

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67
Q

3 factors in virchows triad

A

hyper coagulability
venous stasis
endothelial injury

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68
Q

what does virchows triad do?

A

describes 3 factors that are important in the development of a venous thrombus

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69
Q

what is venous stasis

A

loss of proper vein function of the legs that would usually carry blood back towards the heart

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70
Q

6 risk factor categories for VTE

A
  1. physiological factors (dehydration, obesity, pregnancy, older age)
  2. medications (HRT, some cancer treatments, oestrogen containing contraception)
  3. cancer (cancer type, stage)
  4. VTE and thrombophillia (VTE personal history, first degree relative with VTE, thrombophillia (inherited or acquired))
  5. hospitalisation/ surgery (surgery with general anaesthetic >90mins, critical care admission, hospital admission especially infection, inflammation, reduced mobility, etc. )
  6. other factors (medical comorbidities, significant reduction in mobility, vascular access and devices)
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71
Q

3 different clinical circumstance of VTE

A
  1. provoked by transient significant factor within the last 3 months (such as recent hospitalisation/surgery, pregnancy, oestrogen therapy, lower limb immobilisation)
  2. provoked by transient minor risk factor (e.g. long haul flight)
  3. unprovoked (no obvious precipitant)
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72
Q

Steps to diagnose DVT

A
  • two level DVT wells score
  • D-dimer (screening tool)
  • Confirmatory tests (venous ultrasonography, MRV or CTV for VTE at unusual sites)

(MRV - magnetic resonance venography- used contrast dye to visualise veins)
(CTV - computed tomography venography)

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73
Q

where are the unusual sites for a VTE

A

upper limb
cerebral
portal or hepatic vein
IVC or SVC

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74
Q

steps to diagnose a PE

A 
two level PE wells score
D-dimer (Screening tool )
ECG
chest radiograph (CXR)
confirmatory tests: (CTPA - computer tomography pulmonary angiogram, VQ scan)
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75
Q

what can give you a false negative D dimer

A

if someone is on anticoagulants

late VTE presentation

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76
Q

what is thrombophilia

A

condition that increases your risk of blood clots

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77
Q

inherited causes of thrombophilia

A
  • Factor V Leiden (most common)
  • prothrombin gene mutation
  • natural anticoagulant deficiencies (antithrombin, protein C and S - all rare)
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78
Q

what to do if suspected acquired thrombophilia

A

screen for antiphospholipid syndrom

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79
Q

what is antiphospholipid syndrome

A

a condition in which the immune system mistakenly creates antibodies that attack tissues in the body. These antibodies can cause blood clots to form in arteries and veins.

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80
Q

6 clinical features of antiphospholipid syndrome

A
  1. arterial thrombosis
  2. VTE
  3. microvascular thrombosis
  4. pregnancy loss (recurrent early or late)
  5. livedo reticularis
  6. thrombocytopenia

livedo reticularis - refers to various conditions in which there is mottled discolouration of the skin.

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81
Q

VTE treatment

A

DOAC - direct oral anticoagulants

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82
Q

4 most common clinical indications for anticoagulants

A
  1. VTE treatment (therapeutic dose)
  2. VTE prevention ( lower prophylactic dose)
    - Primary (in hospital)
    - Secondary (after 3-6 months treatment after VTE)
  3. stroke prevention in AF (therapeutic dose)
  4. mechanical heart valves (therapeutic dose)
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83
Q

LMWH stands for?

A little bit about it…

A

low molecular weight heparin
derived from unfractionated heparin
acts longer and more predictably in the body than UFH
can be administered at home (subcut.) and doesn’t require monitoring like UFH

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84
Q

UFH stands for?

a little bit about it..

A

unfractionated heparin
fast acting blood thinner
binds to antithrombin and enhances bodies ability to inhibit factors xa and IIa (most potent clot forming factors)
it doesn’t breakdown clots but stop them growing and new ones forming
rapidly reversed by protamine

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85
Q

what is UFH reversed by

A

protamine

With UFH meaning unfractionated heparin

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86
Q

what is warfarin

A

oral anticoagulant

a coumarin derivative

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87
Q

how does warfarin work

A

inhibits vitamin K metabolism

reduces levels of vitamin K dependent clotting factors II, VII, IX, X

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88
Q

describe a bit about warfarin

onset and half life, causes increased what? monitoring using? what is it reversed with?

A

slow onset and long half life
causes increased PT and APTT
monitoring using INR test (range 2-3)
reversed with vitamin K

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89
Q

When would you prescribe warfarin

A
  • obviously no contra indications
    -mechanical heart valve
    -severe renal failure
    _APLS
    -AF with rheumatic mitral stenosis
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90
Q

mechanism of warfarin

A

inhibitor of vitamin K - dependent factors

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91
Q

mechanism of Apixaban (DOAC)

A

direct inhibitor of Xa factor

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92
Q

mechanism of dabigatran (DOAC)

A

direct thrombin inhibitor

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93
Q

mechanism of rivaroxaban (DOAC)

A

direct factor Xa inhibitor

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94
Q

mechanism of edoxaban (DOAC)

A

direct factor Xa inhibitor

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95
Q

known risk factors for anticoagulant related bleeding

A 
previous major bleed
increasing age
renal impairment
uncontrolled hypertension
concurrent anti platelet medication
excess alcohol
high risk of injury
bleeding disorder
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96
Q

rank the renal clearance of these drugs from highest to lowest:
Warfarin, Apixaban , dabigatran, rivaroxaban , edoxaban

A 
Dabigatran (88%)
Edoxaban (50%)
Rivaroxaban (33%)
Apixaban (27%)
Warfarin (8%)
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97
Q

rank the half life of these drugs from longest to shortest:

Warfarin, Apixaban, Dabigatran, Rivaroxaban, Edoxaban

A 
Warfarin (40hrs)
Dabigatran (12-18 hrs)
Apixaban (12hrs)
Edoxaban (10-14hrs)
Rivaroxaban (5-9hrs in young, 11-13 elderly)
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98
Q

Are these things more common in primary haemastosis disorders or coagulation disorders?

  • Haemarthrosis (bleeding into joints)
  • Haematoma (bleeding into soft tissue)
  • Bruises/petechiae/purpura/eccyhymosis
  • epistaxis, oral bleeds (nose and mouth)
  • heavy menstrual bleeding
  • obstetric and surgical bleeding
  • CNS and gut
A
  • Haemarthosis: Coagulation disorder
  • Haematoma: Coagulation disorder
  • Bruises/petechiae:primary haemastosis disorders
  • Epistaxis:primary haemastosis disorders
  • Heavy menstrual bleeding: both
  • Obstetric and surgical: both
  • CNS and gut: both
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99
Q

What is ISTH score used for

A

used to determine DIC (disseminated intravascular coagulation)

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100
Q

What deficiency does haemophilia A have?

A

factor VIII

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101
Q

what deficiency does haemophilia B have?

A

Factor IX

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102
Q

Haemophilia clinical and laboratory features

A

soft tissue and joint bleeding (haematoma/haemarthosis)
bleeding after surgery and dental extraction
lab results:
increased APTT but normal PT and PLT
decreased factor VIII OR factor IX

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103
Q

what happens in terms of haemostasis in liver disease

A
  • decreased clotting factors, fibrinogen and regulator levels because of reduced protein synthesis
  • decreased clotting factor function because of vitamin K malabsorption in cholestasis
  • decreased PLT numbers because of reduced thrombopoietin synthesis
  • decreased PLT function because of metabolic disturbance
  • increased fibrinolysis
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104
Q

what is cholestasis

A

decrease in bile flow due to impaired secretion by hepatocytes or to obstruction of bile flow through intra-or extrahepatic bile ducts. Therefore, the clinical definition of cholestasis is any condition in which substances normally excreted into bile are retained.

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105
Q

what is PLT count

A

count of platelets

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106
Q

clinical and lab features of liver disease (thinking ABC cycle)

A 
bleeding in skin, soft tissue and GI tract
lab results:
increased PT and APTT
decreased fibrinogen
decreased platelet count (PLT)
increased D dimer
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107
Q

lab features of patient in DIC

A

increased PT and APTT (partial thromboplastin time)
decreased platelet count (PLT)
++ increased D dimer
Plus: usually anaemia and evidence of organ dysfunction

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108
Q

two pro-haemostatic drugs

A

tranexamic acid - major haemorrhage
Desmopressin (DDAVP) - minor

(allows blood to form clots)

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109
Q

describe tranexamic acid

what does it do and what is it effective in treating

A
  • blocks plasmin binding and activation on fibrin and reduces fibrinolysis
  • effective in genetic and acquired bleeding disorders and in major haemorrhage
110
Q

describe Desmopressin (DDAVP)

A
  • releases endogenous factor VIII and vWF

- effective in mild haemophilia A and vWF deficiency, but also in other mild bleeding disorders

111
Q

3 main indications to use FFP (fresh frozen plasma)

A
  1. bleeding in severe liver disease
  2. massive transfusion
  3. DIC (disseminated intravascular coagulation)
112
Q

4 main indications to transfuse platelet concentrates

A
  1. bone marrow failure
  2. exposure to anti-PLT drugs
  3. Trauma, DIC, massive transfusion
  4. genetic PLT disorders
113
Q

4 risks of transfusion

A
  1. unavailable blood
  2. transfusion transmitted infection
  3. immunological reactions
  4. overloading (iron, fluid)
114
Q

5 steps to achieving blood safety

A
  1. donor selection
  2. testing of blood
  3. pathogen inactivation
  4. appropriate use of blood
  5. Haemoviligance (monitoring system)
115
Q

What to screen in blood donors

A

healthy volunteers
aged 17-70
medically assessed (history, medication, travel)
fingerprick Hb (women > 125, men >135 g/l)

116
Q

Uses of red cell transfusion

A

blood loss

anaemia

117
Q

red cell transfusion (what’s needed.. i.e. shelf life etc.)

A

they have 35 day shelf life at 4 degrees

transfuse < 4 hrs after removing from fridge

118
Q

reasons for using platelet concentrate transfusions

A

thrombocytopenia
bleeding with low plt
preventing bleed (v low plt, before procedures)

119
Q

FFP (what’s needed.. shelf life etc.)

A

plasma frozen to -30 degrees celsius
2 yr storage
for clotting factor replacement if bleeding
pre thawed for major haemorrhage

120
Q

dose of plasma infusion for major haemorrhage

A

1 unit FFP and 2 units RBC

121
Q

alternatives to transfusion

A

treat underlying cause
reduce surgical bleeding (stopping aspirin/anticoags before surgery, pro-coag drugs ex. tranexamic acid)
minimise use of blood (follow transfusion thresholds, transfuse minimum required)
autologous blood transfusion(intraoperative cell salvage)

122
Q

what is major incompatibility in transfusion

A

when the recipient has antibodies against transfused blood (e.g. group A given to group O patient)

123
Q

what is. minor incompatibility in transfusion

A

transfused blood contains antibodies against recipient cells
(e.g. group O blood transfused to group A patient)

124
Q 
recipient blood group O:
antibodies formed
red cells chosen for transfusion
antigens on red cells
plasma chosen for transfusion
A

anti-A and anti-B antibodies
Group O red cell transfusion only
no antigens on red cells
any plasma for transfusion

125
Q 
recipient blood group A:
antibodies formed
red cells chosen for transfusion
antigens on red cells
plasma chosen for transfusion
A

anti-B antibodies
Group A or O red cell transfusion
antigen A on red cells
Group A or AB for plasma transfusion

126
Q 
recipient blood group B:
antibodies formed
red cells chosen for transfusion
antigens on red cells
plasma chosen for transfusion
A

Anti-A antibodies
Group B or O red cell transfusion
Antigen B on red cells
Group B or AB for plasma transfusion

127
Q 
recipient blood group AB:
antibodies formed
red cells chosen for transfusion
antigens on red cells
plasma chosen for transfusion
A

No ABO antibodies
Group A, B, O, AB red cell transfusion
AB antigens on red cells
Group AB for plasma transfusion

128
Q

what is cryoprecipitate used for

A

used to prevent or control bleeding in people whose own blood does not clot properly.

129
Q

layers of normal arterial structure from innermost to outermost

A

Intima (endothelial layer)
internal elastic lamina
media (smooth muscle cells, matrix - elastic, collagen, proteoglycans)
external elastic lamina
adventitia (loose connective tissue, vaso vasorum)

In Ireland Men eat apples

130
Q

sequences in a progression of an atherosclerosis

A
  • initial lesion (histologically normal, macrophage infiltration, isolated from cells)
  • fatty streak (mainly intracellular lipid accumulation)
  • intermediate lesion (intracellular lipid accumulation, small extracellular lipid pools)
  • atheroma (intracellular lipid accumulation, core of extracellular lipid)
  • firboatheroma ( single or multiple lipid cores, fibrotic/calcific layers)
  • complicated lesions (surface defect, haematoma-haemorrhage, thrombosis)
131
Q

risk factors for atherosclerosis

A 
high cholesterol and triglyceride levels
high BP
smoking
diabetes
obesity
eating saturated fats
132
Q

main diseases/conditions caused by atherosclerosis

A

stroke

MI

133
Q

novel markers of atherosclerosis

A

high sensitivity CRP
homocysteine
lipoprotein A

134
Q

heart consequences of atherosclerosis

A

angina
ACS (plaque rupture, plaque erosion, calcific nodule)
AF

135
Q

Brain consequences of atherosclerosis

A 
cerebrovascular accident (CVA/TIA/ Stroke) - carotid stenosis, thromboembolic, haemorrhage
vascular dementia
136
Q

peripheral consequences of atherosclerosis

A

renal artery stenosis
aortic aneurysm
peripheral arterial disease ( claudication, acute ischaemia)
impotence (erectile dysfunction)

137
Q

what happens in acute thrombosis

A 
plaque disruption
collagen exposure
platelet activation
platelet aggregation
vessel occlusion
138
Q

management of acute St elevation MI therapy

A

thrombolysis (pre-hospital or hospital)

PCI - percutaneous coronary intervention (primary or facilitated)

139
Q

drugs to reduce platelet activation

A

aspirin
P2Y12 receptor antagonist (either irreversible: ticlopidine, clopidogrel, prasugrel or reversible: ticagrelor, cangrelor)
𝝰IIb𝛃3 antagonist (abciximab, tirofiban)

140
Q

clinical use of drugs to reduce platelet activation

A 
acute MI
In patients with risk of MI
following coronary bypass
unstable coronary syndrome
following coronary artery angioplasty
TIA or thrombotic stroke
141
Q

side effects of drugs used to reduce platelet activation

A

oral drugs - GI disturbances, indigestion
bleeding - GI, nose bleeds, bruising, major bleeding
SOB - ticagrelor specifically

142
Q

what are fibrinolytic drugs

A

the clot busterrrs

143
Q

examples of fibrinolytic drugs

A
  • steptokinase : from streptococci , forms complex with plasminogen to produce plasmin, 1 yr must elapse before reuse, cheap
    -recombinant tissue plasminogen activator (tPA): Alteplase ; more active at fibrin bound plasminogen so “clot” specific. short half life so iv infusion
    Reteplase; longer half-life so can give as IV bolus / penetrate inside thrombus more clot specific

(fibrinolytic drug, also called thrombolytic drug, any agent that is capable of stimulating the dissolution of a blood clot (thrombus).

144
Q

clinical use of fibrinolytic drugs

A

in acute MI within 12 hrs of onset, sooner the better
acute thrombotic stroke
clearing thrombus shunts/cannulae
acute arterial thromboembolism

145
Q

unwanted effects of fibrinolytic drugs

A

GI haemorrhage/stroke (treat with tranexamic acid)
low grade allergic reactions/fever
burst of plasmin generated by steptokinase can generate kinins causing hypotension

146
Q

contraindications of fibrinolytic drugs (absolute and relative)

A

absolute:
-active or recent internal bleeding
-recent cerebrovascular event
-invasive procedures where haemostasis is important
relative:
-trauma
-pregnancy
-peptic ulcers
-bacterial endocarditis

147
Q

antifibrinolytic drugs examples

A
  • tranexamic acid (inhibits plasminogen activity, oral or IV administration, clinical use when increased risk of bleeding, side effects of nausea and vomiting)
  • aprotinin (proteolytic enzyme inhibitor of plasmin, slow iv, used in patients with high risk of blood loss, usually well tolerated - no side effects)
148
Q

define anaemia

A

a condition in which there is a deficiency of red cells or of haemoglobin in the blood, resulting in pallor and weariness.

149
Q

3 general symptoms of anaemia

A

fatigue
dyspnoea - SOB
headaches

150
Q

history to elicit when seeking cause of anaemia

A

blood loss
weight loss
diet
comorbidities and medications

151
Q

general examination features of anaemia

A 
pallor 
peripheral oedema
tachycardia/arryhtmias
flow murmur
SOB
confusion (especially in elderly)
152
Q

investigations for anaemia

A

FBC needed to confirm diagnosis, MCV will guide further tests
blood films and haemanitics , renal function, LFTs,

153
Q

microcytic anaemia

A

MCV <83fL
iron deficiency
thalassaemia
anaemia of chronic disease

(ita like pobrecITA smalllll)

154
Q

Normocytic anaemia

A 
MCV 83-96fL
acute blood loss
haemloysis
anaemia of chronic disease
bone marrow infiltration
combined haeminitic deficiency
155
Q

macrocytic anaemia

A 
MCV>96fL 
B12/folate deficiency 
haemolysis
hypothyroidism
liver diseae
alcohol excess
myelodysplasia
156
Q

what are haeminitic deficiencies

A

iron
vitamin B12
folate

157
Q

Describe iron absorption and amount needed

A

total body iron should be 4g (Hb 3g, RE cells 1g)

Fe absorption through duodenum

158
Q

causes of iron deficiency

A

dietary (80% from meat, 20% from vegetables)
physiological (infancy, adolescence, pregnancy)
blood loss (most common cause in UK)
malabsorption (e.g. coeliac disease)

159
Q

clinical features of iron deficiency

A
  • angular stomatitis (red swollen patches in corners of mouth)
  • sore mouth
  • Koilonychia (spoon nails)
  • pharyngeal and oesophageal webs
160
Q

laboratory features of iron deficiency

type of anaemia, low serum what? elevated what?

A
  • microcytic hypochromic anaemia (hypochromic meaning RBCs have less Hb than normal)
  • low serum ferritin (beware:acute phase protein)
  • low serum iron
  • elevated TIBC (total iron binding capacity) and serum transferrin saturation
  • absent iron stores in bone marrow
161
Q

talk about Vitamin B-12 (daily requirement, body stores, synthesised by, absorption, type of anaemia)

A

daily requirement: 1-2µg
body store: 2-3mg mainly in liver
synthesised by micro-organisms: only present naturally in animal products
absorption: combines with intrinsic factor secreted by gastric parietal cells and absorbed in terminal ileum
megaloblastic anaemia (bone marrow produces unusually large, structurally abnormal, immature red blood cells) (macrocytic anaemia)

162
Q

causes of vitamin B-12 deficiency

A
  • dietary: veganism, rare
  • intrinsic factor deficiency: pernicious anaemia (an autoimmune condition that affects your stomach, most common in UK), gastrectomy, congenital
  • intestinal malabsorption: disease of terminal ileum(e.g. chrons), blind loops and small bowel diverticulae
163
Q

clinical features of vitamin B-12 deficiency

A

jaundice
glossitis ( inflammation of the tongue.)
neurological deficit

164
Q

laboratory features of vitamin B-12 deficiency

antibodies against what? what changes in bone marrow? features of what?

A
  • anaemia, neutropenia (occurs when you have too few neutrophils, a type of white blood cells.), thrombocytopenia (a condition in which you have a low blood platelet count.)
  • low serum vitamin b12
  • antibodies against parietal cells or intrinsic factors
  • megaloblastic change in bone marrow
  • features of haemolysis
165
Q

talk about folate (folic acid) (daily requirement, body stores, dietary sources, where its absorbed, type of anaemia)

A

daily requirement: 100-200 µg
body stores: 10-15mg
dietary sources: green vegetables, liver, nuts, cereals
absorbed in jejunum
megaloblastic anaemia (bone marrow produces unusually large, structurally abnormal, immature red blood cells)

166
Q

causes of folate (folic acid) deficiency

A
  • dietary: common in elderly, poor diet related to alcohol misuse
  • increased utilisation: e.g. in pregnancy, malignancy, haematological disorders with rapid cell turnover
  • malabsorption e.g. coeliac disease
  • drugs e.g. anticonvulsants
  • excessive loss e.g. renal dialysis
167
Q

clinical features of folate (folic acid) deficiency

A 
extreme tiredness
a lack of energy
pins and needles (paraesthesia)
a sore and red tongue
mouth ulcers
muscle weakness
disturbed vision
psychological problems, which may include depression and confusion
problems with memory, understanding and judgement

similar to vitamin B-12 deficiency but neurological symptoms do not occur
(jaundice
glossitis ( inflammation of the tongue.))

168
Q

laboratory features of folate (folic acid) deficiency

A

peripheral blood and bone marrow features identical to vitamin B-12 deficiency
diagnosis made by measuring low serum(and sometimes red cell) folate levels

169
Q

treatment of B12/folate deficiency

A
  • consider underlying cause
  • vitamin B12 replacement as IM injection most common: 3x per week or alternate days for 2 weeks then maintenance phase
  • oral folic acid replacement
  • care: risk worsening neurological symptoms if folic acid is supplemented in b12 deficiency: check both and if in doubt replace B before F
170
Q

RBC breakdown can be…. (location)

A

intravascular (within the vessels releasing free Hb)

extravascular (by reticuloendothelial system)

171
Q

describe inherited haemolytic anaemia

membrane causes, enzyme causes, haemoglobin causes

A
  • membrane: hereditary spherocytosis (autosomal-dominant)
  • enzymes: glucose 6 phosphate dehydrogenase deficiency (X linked recessive), pyruvate kinase deficiency (autosomal recessive)
  • haemoglobin: sickle cell anaemia, thalassaemia(autosomal recessive)
172
Q

describe causes of acquired haemolytic anaemia

A
  • Immune: i)autoimmune (primary or secondary to lymphoproliferative, autoimmune disorders, drugs, infections) ii) alloimmune (e.g. haemolytic disease of the newborn, incompatible blood transfusion)
  • infections: many mechanisms
  • drugs and chemicals: many mechanisms
  • mechanical: MAHA (microangiopathic haemolytic anaemia) prosthetic heart valves
173
Q

alloimmune meaning in anaemia

A

red blood cells and immune system don’t match

174
Q

7 clinical features of haemolytic anaemia

A

1) anaemia
2) jaundice
3) splenomegaly
4) skeletal abnormalities (congenital forms)
5) gallstones (pigment stones suggests chronic haemolysis)
6) dark urine (increased urobilnogen)
7) haemoglobinuria (denotes intravascular haemolysis)

175
Q

what is reticulocytosis

and what it could indicate

A

high reticulocyte count
this is when there is an increase in production of red blood cells to compensate for the loss of mature red blood cells. This could indicate: hemolytic anemia, acute bleeding, chronic blood loss or a kidney disease.

176
Q

what is polychromasia

A

occurs on a lab test when some of your red blood cells show up as bluish-gray when they are stained with a particular type of dye. This happens when red blood cells are immature because they were released too early from your bone marrow.

177
Q

What indicates microangiopathic haemolytic anaemia (MAHA)

A

red cell fragments (schistocyte)

broken down within the micro-vasculature (intravascular

178
Q

causes of microangiopathic haemolytic anaemia (MAHA)

A
  • thrombotic thrombocytopenic purpura (TTP)
  • haemolytic uremic syndrome (HUS)
  • DIC
  • Malignant hypertension
  • Vasculitis
  • metastatic adenocarcinoma
179
Q

what are haemoglobinopathies?

two common examples

A
  • inherited genetic defects of globin
  • sickle cell anaemia and thalassaemias are most clinically important
  • mutations of the ɑ globin genes affect both foetal and adult life
  • sickle cell anaemia is an altered βglobin protein structure
180
Q

3 clinical types of beta thalassemia

A
  • beta thalassemia trait (Carrier): β0/β0
    asymptomatic and normal life span
    (where β - normal beta globing gene and β0- non functioning beta globin gene)

-beta thalassemia intermedia
variable genotype, phenotype, and life span

-beta thalassemia major: β0/β0
no beta globin and therefore no HbA

181
Q

beta thalassemia major (β0/β0) pathophysiology

A
  • complete absence of HbA (as no β globin)
  • excess ɑ chains accumulate and damage red cells
  • ineffective erythropoiesis
  • excessive RBCs destruction
  • iron overload (increased absorption and transfusion dependence)
  • extra-medullary haematopoeisis
182
Q

beta thalassemia major clinical features

A
  1. asymptomatic at birth
  2. symptomatic anaemia in the first few months of life
  3. jaundice
  4. growth retardation failure to thrive
  5. medullary hyperplasia - bony abnormalities especially on the facial bones
  6. extra-medullary haematopoiesis - enlarged liver and spleen
  7. increased risk of thromboses
  8. pulmonary hypertension and congestive heart failure
183
Q

treatment of beta thalassemia major

A
  1. regular blood transfusions (life long)
  2. iron chelation ( drugs that help the body excrete iron)
  3. folic acid
  4. bone marrow transplantation
184
Q

ɑ thalassemia silent carrier explained

A

one gene not working
asymptomatic
minority showing reduced MCV and MCH

185
Q

ɑ0 thalassemia trait

ɑ+/ɑ+ thalassemia trait both explained

A 
two genes not working
Hb normal or slightly reduced 
MCV and MCH reduced 
no symptoms 
for both phenotype is indistinguishable
186
Q 
HbH  disease
(what is it? how many genes don't work? how would someone present? what is the treatment?)
A

type of ɑ thalassemia
three genes not working
decreased ɑ chains
display: jaundice, hepatosplenomegaly, leg ulcers, gallstones
treatment: folic acid, occasional transfusions, +/- splenectomy

187
Q

Hb Barts hydrops

A 
type of ɑ thalassemia
four genes not working
no ɑ chains produced
mainly ɣ chains in utero: form tetramers
intrauterine death and stillborn
188
Q

normal vs. sickle red cells

A

normal:
disc-shaped
deformable
life span of 120 days

sickle:
sickle-shaped
rigid
lives away for 20 days or less

189
Q

3 points on sickle cell anaemia

A

haemolysis: chronic anaemia, increased reticulocytes, increased LDH and bilirubin

vaso-occlusion: acute episodes and chronic deterioration

hyposplenism: (reduced function of the spleen) risk of infection with encapsulated bacteria

190
Q

acute complications of sickle cell anaemia

A 
hand-foot syndrome
splenic sequestration
infections
acute chest syndrome
stroke
venous thrombosis
priaprism
aplastic crisis (pavovirus)
191
Q

what is priaprism

A

persistent and painful erection of the penis.

192
Q

chronic complications of sickle cell anaemia

A 
delayed growth and puberty in children
gallstones
blindness
kidney failure
leg ulcers
pulmonary arterial hypertension 
hip problems - avascular necrosis
193
Q

types of genetic damage

in anaemia cycle

A 
point mutations
chromosomal translocations
chromosomal inversions
chromosomal deletions
chromosomal duplication
epigenetic alterations 
microRNAs
194
Q

Cancers in haematology

A 
acute lymphoblastic leukaemia 
CLL (chronic lymphoblastic leukaemia) and lymphomas
myeloma
acute myeloid leukaemia 
CML  + myeloproliferative disorders
195
Q

brief description of term leukaemia

A

can be myeloid or lymphoid, acute or chronic
“runny” cancer of white cells
predominately affects bone marrow and blood

196
Q

brief description of term lymphoma

A

only lymphoid, Hodgkins or non-Hodgkins, high grade or low grade, B or T cells
“solid” cancer of lymphocytes
predominately affects lymph nodes but can be extra nodal

197
Q

brief description of myelodysplasia

A

bone marrow dysfunction

198
Q

brief description of myeloproliferative neoplasms

A

increased number of normal myeloid cells (RBC, platelets, neutrophils)

199
Q

brief description of myeloma

A

cancer of plasma cells within the bone marrow

200
Q

difference between acute and chronic leukaemia

A

acute - undifferentiated

chronic - differentiated

201
Q

how may haematological cancer present

A

incidental abnormal FBC or other blood test
lymphadenopathy
splenomegaly
symptoms due to too few cells (anaemia symptoms, bleeding/bruising, infection/fever)
symptoms due to too many cells
constitutional symptoms (weight loss, lethargy, itching, fever, sweats)
Other: bone pain or pathological fracture, hypercalcaemia, renal failure, mass due to lymphoma

202
Q

what does lymphadenopathy mean

A

swollen lymph nodes

203
Q

if myeloma suspected what tests would you do

A 
immunoglobulins 
SPE (Serum protein electrophoresis)
serum free light chains
BJP (Bence jones protein)
renal function
calcium
204
Q

if lymphoma suspected what laboratory tests would you do

A

lymph node biopsy

imaging (CT, MRI , PET scan)

205
Q

What does LDH look at

A

its a marker of cell turnover

206
Q

what does a grade of cancer mean

A

how aggressive the cancer is

207
Q

what does stage of cancer mean

A

how far along the cancer is

208
Q

5 treatment options for haematological cancers

A
  • supportive care (blood transfusions, infection management, etc.)
  • chemotherapy (not specific, also kills healthy cells)
  • radiotherapy (some lymphomas or myeloma local symptoms)
  • targeted therapies aimed at specific molecular targets (includes immunotherapies)
  • haematopoetic stem cell transplant HSCT
209
Q

briefly cover splenomegaly in anaemia

A
  • typically causes no symptoms
  • Pain or fullness in left upper belly that can spread to the left shoulder
  • a feeling of fullness without eating or after eating a small amount because the spleen is pressing on your stomach
  • splenomegaly from any cause can lower blood cell numbers (hypersplenism)
  • can palpate on an abdominal exam
  • confirm with USS
210
Q

causes of splenomegaly

A
  • infections
  • cirrhosis of the liver and other liver diseases
  • haemolytic anaemia (early destruction of RBCs)
  • blood cancers, such leukaemia and myeloproliferative neoplasms, and lymphomas, such as Hodgkins disease
  • pressure on the veins in the spleen or liver or a blood clot in these veins
  • autoimmune conditions such as lupus or sarcoidosis
211
Q

what is sarcoidosis

A

is a disease characterized by the growth of tiny collections of inflammatory cells (granulomas) in any part of your body — most commonly the lungs and lymph nodes. But it can also affect the eyes, skin, heart and other organs

212
Q

reactive causes of lymphadenopathy

A

infection
non haematological cancer (breast cancer)
inflammatory

213
Q

clonal causes of lymphadenopathy

A

lypmhoma

leukaemia

214
Q

where to look for lymphadenopathy

A 
neck
axilla
inguinal
liver
spleen
215
Q

how to assess lymphadenopathy

A
  • clinically divide into localised (one lymph node group) or generalised (several lymph node groups)
  • if localised is there a local infective or neoplastic cause?
  • duration of lymph node enlargement and any change in size?
  • any accompanying B symptoms (>10% weight loss in 6 months, soaking sweats, unexplained fevers?)
  • any hepatosplenomegaly, lymphocytosis, cytopenias
216
Q

when is it abnormal lymphadenopathy

A

-lymphadenopathy >1cm persisting for >6weeks with no obvious infective precipitant
-small volume inguinal lymphadenopathy is a common normal finding. refer if >2cm
-lymphadenopathy for <6 weeks associated with:
B symptoms, rapidly enlarging nodes, generalised lymph, splenomegaly or hepatomegaly, anaemia, thrombocytopenia, or neutropenia , hypercalcaemia

217
Q

out of Hodgkins and non-Hodgkins lymphoma which is more common

A

Non-Hodgkins

218
Q

is a low grade non-Hodgkins lymphoma more or less curable than a high grade non-hodgkins lymphoma

A

low grade is slow growing and less curable

high grade is fast growing and more curable

219
Q

clinical presentation of lymphomas

A
  • usually presents with enlarged lymph nodes
  • B symptoms
  • bone marrow suppression due to infiltration
  • enlarged spleen or liver
  • lymphocytosis
  • itching
  • extranodal lymphoma (e.g. gut, thyroid, parotid, breast, ovary, testis, CNS etc.)
220
Q

brief description of Hodgkin’s lymphoma

incidence, presence of what cell, presents with, histological appearance

A
  • first peak incidence in young adults (20-30yrs), second peak in the elderly
  • Presence of Reed-Sternberg cell (B-cell origin)
  • usually presents with lymphadenopathy
  • May have B symptoms
  • may have itching
  • defined by histological appearance (looks like owl eyes)
  • multinucelated
221
Q

brief description of Non-Hodgkins lymphoma (NHL)

Which is more common B or T cell? Risk factors? High grade means? Low grade means?

A
  • more common than Hodgkins
  • B cell derived NHL more common than T cell derived NHL
  • Risk factors include: HIV, EBV, and immunosuppression
  • similar staging to Hodgkins
  • High grade: short history, fast growing, more curable, needs urgent treatment
  • Low grade: slow growing, more indolent (causing little or no pain), less curable, may not need treatment and can be observed
222
Q

what does AML stand for (in anaemia block)

A

acute myeloid leukaemia

223
Q

What does ALL stand for (in anaemia block)

A

acute lymphoblastic leukaemia

224
Q

brief overview of acute leukaemias

A
  • proliferation of WBC “blasts”
  • accumulate in the bone marrow
  • reduced bone marrow production of healthy blood cells
  • present with symptoms of reduced production of normal red cells, platelets and neutrophils: anaemia, bleeding, bruising, infection
  • general cancer symptoms (weight loss, tiredness, sweats, fevers)
  • Acute lymphoblastic leukaemia may have lymphoid mass
  • patients have a relatively short history and are often unwell
225
Q

AML incidence

A

acute myeloid leukaemia incidence increases with age

226
Q

ALL incidence

A

acute lymphoblastic leukaemia is a disease of the young

227
Q

how to diagnose acute leukaemias

A
  1. FBC results (may be cytopenias)
  2. Blood film: blasts, Auer rods (granules clumped together -myeloid sign)
  3. Bone marrow: morphology, flow cytometry (differentiate between myeloid and lymphoid precursors). cytogenetics and molecular markers
228
Q

CML stands for

A

chronic myeloid leukaemia

229
Q

brief description of chronic myeloid leukaemia (CML)
common in who, characterised by what, presents with..
can progress to…

A
  • commoner in adults
  • characterised by proliferation WITH differentiation (unlike AML) of myeloid leukocytes including mature neutrophils
  • Presentation: High white count (may be detected incidentally)
  • may have constitutional symptoms and splenomegaly
  • can progress to acute leukaemia’s (either AML or ALL)
230
Q

what does CLL stand for and a brief description of it

who is it most common in? cell origin? rate of growth? increased what on FBC? staged according to? treatment?

A

Chronic lymphocytic leukaemia

  • most common leukaemia in Western world
  • most common in elderly
  • B-cell origin, small mature lymphocytes on blood film and smear cells
  • Slow growing, often asymptomatic/incidental findings
  • increased lymphocyte count on FBC
  • staged according to lymphocytosis, lymphadenopathy, enlarged spleen/liver, anaemia or thrombocytopenia
  • many patients never need treatment
231
Q

what does MGUS stand for

A

monoclonal gammopathy of undetermined significance

232
Q

Brief overview of MGUS

A
  1. low level of paraprotein
  2. a low level of abnormal plasma cells in the bone marrow
  3. no indicators of active disease
  • all patients with active myeloma once had MGUS
  • only 20% of patients with MGUS progresses to active myeloma
  • MGUS patients do not need treatment
233
Q

brief overview of Myeloma

more common in who? accumulation of what? asymptomatic? symptomatic?

A
  • more common in elderly
  • incidence 12 per 100,000
  • more common in afro-carribean origin
  • accumulation of neoplastic plasma cells in the bone marrow
  • asymptomatic myeloma: no indicators of active disease (CRABI) but higher level of paraprotein and more bone marrow plasma cells than MGUS
  • symptomatic myeloma: one or more indicators of active disease (MGUS)

CRABI: calcium elevation, renal dysfunction, anaemia and bone disease

234
Q

what is a paraprotein

A

protein found in the blood only as a result of cancer or other disease.

235
Q

symptomatic myeloma presentation (CRABI acronym)

A 
C - hypercalcaemia 
R - renal dysfunction
A - anemia
B - bone - lytic lesions, fractures, osteoporosis
I - infections
236
Q 
CASE (anaemia case cycle):
84 yo asymptomatic
persistent stable lymphocytosis
otherwise normal FBC
No lymphodenopathy or splenomegaly
Blood film - small immature lymphocytes, smear cells and otherwise normal
diagnosis?
A

CLL

chronic lymphocytic leukaemia

237
Q 
CASE (anaemia case cycle):
5 yo boy
malaise, bone pain, fever, rash
FBC: anaemia, neutropenia, thrombocytopenia and high WCC
blood film: circulating blasts 
diagnosis?
what if 60yo man and granules in blasts?
A

Child: ALL - acute lymphoblastic leukaemia
Adult: AML - acute myeloid leukaemia

238
Q 
CASE (Anaemia case cycle):
72 yo man
back pain, renal impairment
femur fracture, no trauma
normocytic anaemia 
hypercalcaemia
lytic lesions on XR
Diagnosis?
A

myeloma

239
Q

what are lytic lesions?

A

An osteolytic lesion is a softened section of a patient’s bone formed as a symptom of specific diseases, including breast cancer and multiple myeloma. This softened area appears as a hole on X-ray scans due to decreased bone density, although many other diseases are associated with this symptom

240
Q

CASE (Anaemia case cycle):
55yo woman
fatigue and reduced appetite
splenomegaly
FBC: normal Hb and platelets, very high WCC
blood film: increased neutrophils, other myeloid leukocytes at varying maturity
Diagnosis?

A

CML

chronic myeloid leukaemia

241
Q

CASE (anaemia case cycle):
28yo man
recent history of progressive cervical lymphadenopathy over weeks, now 5cm
Fevers, drenching night sweats, weight loss, itching
FBC normal
lactate dehydrogenase high
diagnosis

A

High grade NHL (non-hodgkins lymphoma)

Burkitt - highest rate of growing cancer

242
Q

Leukaemia type summary

A

can be Myeloid: can be acute (AML) or chronic (CML)

or can be Lymphoid: can be acute (ALL) or chronic (CLL)

243
Q

types of lymphomas

A

can be Hodgkins

can be Non-Hodgkins: which can be high grade (either T cell or B cell) or low grade (either T cell or B cell)

244
Q

3 types of myeloproliferative neoplasms

A

PRV (polycythaemia Rubra Vera)
ET (Essential thrombocytopenia)
PMF (primary myelofibrosis)

245
Q

what is myelodysplasia

A

Myelodysplastic syndromes are a group of cancers in which immature blood cells in the bone marrow do not mature or become healthy blood cells. The different types of myelodysplastic syndromes are diagnosed based on certain changes in the blood cells and bone marrow

246
Q

difference between monoclonal and polyclonal antibodies

A

Polyclonal antibodies are made using several different immune cells. They will have the affinity for the same antigen but different epitopes, while monoclonal antibodies are made using identical immune cells that are all clones of a specific parent cell

247
Q

Terminology for too many platelets? too few?

A

too few: thrombocytopenia

too many: thrombocytosis

248
Q

terminology for too few neutrophils? too many?

A

too few: neutropenia

too many: neutrophilia

249
Q

terminology for too few lymphocytes? too many?

A

too few: lymphopenia

too many: lymphocytosis

250
Q

terminology for too few RBCs (haemoglobin)? too many?

A

too few: anaemia

too many: polycythaemia

251
Q

reactive causes of neutrophilia

A 
infection (esp. bacteria)
inflammation 
ischaemia, infarction
smoking
splenectomy
pregnancy
corticosteroids
252
Q

clonal causes of neutrophilia

A 
myeloproliferative neoplasms (MPN)
chronic myeloid leukaemia
253
Q

brief overview of myeloproliferative neoplasms (MPN)

A
  • characterised by excessive proliferation of terminally differentiated myeloid cells (normal differentiation ) - cells look normal
  • due to Somatic acquired mutations in haematopoetic stem cells
  • lead to cytokine independent restricted growth
254
Q

Clinical features of MPN (myeloproliferative neoplasms)

A
  • incidental finding of an abnormal blood test result (without symptoms)
  • thrombosis (arterial or venous), occasionally at an unusual site
  • itching, constitutional symptoms (weight loss, fatigue, etc.), increased risk of gout
  • symptoms of splenomegaly (more common with PMF) e.g. early fullness
255
Q

what is treatment of MPN usually aimed at?

A

mainly aimed at reducing risk of blood clots

256
Q

reactive causes of lymphocytosis?

A

infection (esp. viral or chronic bacterial)
inflammation
smoking
splenectomy

257
Q

clonal causes of lymphocytosis?

A

lymphoproliferative (chronic lymphocytic leukaemia most common)

258
Q

what does flow cytometry measure in haematological cancer

A

1) cell type: is it lymphoid, myeloid or non haematological?
2) cell lineage: is it B lymphocyte or T lymphocyte?
3) cell maturity: is it an immature precursor or a more mature cell
4) aberrant antigen patterns which may be specific for some cancers

259
Q

reactive causes of thrombocytosis

A 
trauma (e.g. by surgeon)
infection
inflammation
non haematological malignancy
iron deficiency
splenectomy
260
Q

clonal causes of thrombocytosis

A

myeloproliferative neoplasms (MPN): PRV, ET, PMF

261
Q

what does TTP stand for

A

thrombotic thrombocytopenic purpura

262
Q

5 clinical features of thrombotic thrombocytopenic purpura

A
  1. fever
  2. neurological signs
  3. Renal failure
  4. Low platelets
  5. MAHA (microangiopathic haemolytic anaemia)
263
Q 
thrombocytopenia causes
(broken into non pathological, production, consumption and complex)
A

Not pathological -

  • spurious: lab artefact e.g. platelet clumping
  • physiological: gestational in pregnancy

Production -

  • genetic/inherited: no previous normal results, may have FHx
  • substrate deficiency: B12 or folate deficiency can cause any cytopenia
  • growth factor deficiency: Liver TPO (thrombopoeitin)
  • bone marrow production problem: infiltration, aplasia, dysplasia, fibrosis

consumption-

  • reduced survival / consumption : immune destruction or non immune includes MAHA, DIC)
  • Loss (Bleeding): only anaemia unless massive haemorrhage/transfusion
  • splenomegaly: hypersplenism

complex

  • medications
  • infections
264
Q

neutropenia causes

including non pathological, production, consumption and complex

A

non-pathological-
-physiological: ethnic neutropenia

Production-

  • genetic/inherited: no previous normal results, may have FHx
  • Substrate deficiency: B12 or folate deficiency can cause any cytopenia
  • bone marrow production problem: infiltration, aplasia, dysplasia, fibrosis

Consumption-

  • reduced survival /consumption: immune deficiency, non immune
  • Loss (bleeding): only anaemia unless massive haemorrhage/transfusion
  • splenomegaly: hypersplenism

Complex -

  • medications
  • infections
265
Q

What does TACO stand for

A

transfusion associated circulatory overload

266
Q

describe transfusion associated circulatory overload (TACO)

A

is a common transfusion reaction where pulmonary oedema due to excess volume or circulatory overload results in the patient experiencing acute respiratory distress.

267
Q

What does TRALI stand for

A

transfusion related acute lung injury

268
Q

Describe transfusion related acute lung injury (TRALI)

A

is a rare but serious syndrome characterized by sudden acute respiratory distress following transfusion.

The typical presentation of TRALI is the sudden development of shortness of breath, severe hypoxemia (O2 saturation <90% in room air), low blood pressure, and fever that develop within 6 hours after transfusion and usually resolve with supportive care within 48 to 96 hours.

269
Q

signs and symptoms of TACO

A

appears as acute respiratory distress that begins during or shortly after the administration of a blood product. Symptoms and signs of TACO include dyspnea that worsens as pulmonary edema progresses, orthopnea, chest tightness, cough, tachycardia, hypertension, and widened pulse pressure.

270
Q

signs and symptoms of TRALI

A

The typical presentation of TRALI is the sudden development of shortness of breath, severe hypoxemia (O2 saturation <90% in room air), low blood pressure, and fever that develop within 6 hours after transfusion and usually resolve with supportive care within 48 to 96 hours.

CBL Year 2 (14 decks)

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